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Clinical features of secondary pulmonary alveolar proteinosis associated with myelodysplastic syndrome: Two case reports
Ist Teil von
Medicine (Baltimore), 2017-11, Vol.96 (44), p.e8481-e8481
Ort / Verlag
United States: Wolters Kluwer Health
Erscheinungsjahr
2017
Link zum Volltext
Quelle
EZB Free E-Journals
Beschreibungen/Notizen
Pulmonary alveolar proteinosis (PAP) is a rare lung disorder characterized by the abnormal accumulation of alveolar surfactant protein in alveolar spaces. Secondary PAP can result from myelodysplastic syndrome (MDS).
But most reports described a single case; here we reported 2 cases of PAP secondary to MDS. One case developed secondary PAP at the same time as MDS, and the other developed during the course of MDS.
The diagnosis of PAP was made by bronchoalveolar lavage and based on the identification of periodic acid-Schiff-positive proteinaceous material. Chest high resolution CT (HRCT) scans showed variable distribution of ground glass opacities, but crazy-paving appearance was not seen in our 2 cases.
Because the patients' general conditions were poor, whole lung lavage was not used in the 2 cases.
And the 2 cases' prognoses were poor.
In conclusion, pulmonary physicians should suspect the possibility of secondary PAP when they encounter unexplained pulmonary infiltrates with some hematologic or infectious disease that shows diffuse bilateral GGO on an HRCT scan.